What is it about?

Multiple myeloma is a disease related to the proliferation of malignant plasma cells; in the large majority of patients the disease is confined at the level of bone marrow. However, in a minority of patients the malignant plasma cells are localized also outside bone marrow, either at the level of peripheral blood (plasma cell leukemia) or at the level of soft tissues (extramedullary multiple myeloma). These two rare forms of aggressive MM (ultrahigh-risk (uHR) MM as MM leading to death within 24-36 months) are both associated with some molecular features and with a limited response to current treatments.

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Why is it important?

Extramedullary Multple Myeloma did not take advantage the new therapy like the classical multiple myeloma. So far these forms of myeloma have been treated with the same protocols of classical MM and there are not trials specifically dedicated to them. Also the new therapies with CAR-T Cells or Bispecific antibodies have shown a reduced efficacy on them.

Perspectives

Since extramedullary Myelomas are very rare, international clinical trials are necessary to have a number of patients sufficient to perform controlled studies that are statistically valid. On the other hand, biological studies should be increased to better understand the role of adhesion proteins.

Prof. Giuseppe Leone
Universita Cattolica del Sacro Cuore

Read the Original

This page is a summary of: PLASMACELL NEOPLASMS WITH SPREADING IN THE BLOOD AND TISSUES: EXTRAMEDULLARY MYELOMA DISEASE A RARE AGGRESSIVE FORM OF MULTIPLE MYELOMA (First of two parts), Mediterranean Journal of Hematology and Infectious Diseases, December 2024, Institute of Hematology, Catholic University,
DOI: 10.4084/mjhid.2025.005.
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